Article
Proteolysis of mutant huntingtin produces an exon 1 fragment that accumulates as an aggregated protein in neuronal nuclei in Huntington disease.
The Journal of biological chemistry - 19 Mar 2010
Landles Christian, Sathasivam Kirupa, Weiss Andreas, Woodman Ben, Moffitt Hilary, Finkbeiner Steve, Sun Banghua, Gafni Juliette, Ellerby Lisa M, Trottier Yvon, Richards William G, Osmand Alex, Paganetti Paolo, Bates Gillian P
Abstract excerpt
Huntingtin proteolysis has been implicated in the molecular pathogenesis of Huntington disease (HD). Despite an intense effort, the identity of the pathogenic smallest N-terminal fragment has not been determined. Using a panel of anti-huntingtin antibodies, we employed an unbiased approach to generate proteolytic cleavage maps of mutant and wild-type huntingtin in the HdhQ150 knock-in mouse model of HD. We...
Topics
- Animals
- COS Cells
- Calpain
- Cell Nucleus
- Chlorocebus aethiops
- Cytoplasm
- Disease Models, Animal
- Exons
- Genotype
- Huntingtin Protein